Clinical Utility and Validation of the Krakow DCM Risk Score-A Prognostic Model Dedicated to Dilated Cardiomyopathy.

BACKGROUND: One of the most common causes of heart failure is dilated cardiomyopathy (DCM). In DCM, the mortality risk is high and reaches approximately 20% in 5 years. A patient's prognosis should be established for appropriate HF management. However, so far, no validated tools have been available for the DCM population. METHODS: The study population consisted of 735 DCM patients: 406 from the derivation cohort (previously described) and 329 from the validation cohort (from 2009 to 2020, with outcome data after a mean of 42 months). For each DCM patient, the individual mortality risk was calculated based on the Krakow DCM Risk Score. RESULTS: During follow-up, 49 (15%) patients of the validation cohort died. They had shown significantly higher calculated 1-to-5-year mortality risks. The Krakow DCM Risk Score yielded good discrimination in terms of overall mortality risk, with an AUC of 0.704-0.765. Based on a 2-year mortality risk, patients were divided into non-high (≤6%) and high (>6%) mortality risk groups. The observed mortality rates were 8.3% (n = 44) vs. 42.6% (n = 75), respectively (HR 3.37; 95%CI 1.88-6.05; p < 0.0001). CONCLUSIONS: The Krakow DCM Risk Score was found to have good predictive accuracy. The 2-year mortality risk > 6% has good discrimination for the identification of high-risk patients and can be applied in everyday practice.

Extended Precordial T Wave Inversions Are Associated with Right Ventricular Enlargement and Poor Prognosis in Pulmonary Hypertension.

In pulmonary hypertension (PH), T wave inversions (TWI) are typically observed in precordial leads V1-V3 but can also extend further to the left-sided leads. To date, the cause and prognostic significance of this extension have not yet been assessed. Therefore, we aimed to assess the relationship between heart morphology and precordial TWI range, and the role of TWI in monitoring treatment efficacy and predicting survival. We retrospectively analyzed patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) treated in a reference pulmonary hypertension center. Patients were enrolled if they had a cardiac magnetic resonance (cMR) and 12-lead surface ECG performed at the time of assessment. They were followed from October 2008 until March 2021. We enrolled 77 patients with PAH and 56 patients with inoperable CTEPH. They were followed for a mean of 51 ± 33.5 months, and during this time 47 patients died (35.3%). Precordial TWI in V1-V6 were present in 42 (31.6%) patients, while no precordial TWI were observed only in 9 (6.8%) patients. The precordial TWI range correlated with markers of PH severity, including right ventricle to left ventricle volume RVEDVLVEDV (R = 0.76, p < 0.0001). The presence of TWI in consecutive leads from V1 to at least V5 predicted severe RV dilatation (RVEDVLVEDV ≥ 2.3) with a sensitivity of 88.9% and specificity of 84.1% (AUC of 0.90, 95% CI = 0.83-0.94, p < 0.0001). Presence of TWI from V1 to at least V5 was also a predictor of mortality in Kaplan-Meier estimation (p = 0.02). Presence of TWI from V1 to at least V5 had a specificity of 64.3%, sensitivity of 58.1%, negative predictive value of 75%, and positive predictive value of 45.5% as a mortality predictor. In patients showing a reduction in TWI range of at least one lead after treatment compared with patients without this reduction, we observed a significant improvement in RV-EDV and RV-EDVLV-EDV. We concluded that the extension of TWI to left-sided precordial leads reflects significant pathological alterations in heart geometry represented by an increase in RV/LV volume and predicts poor survival in patients with PAH and CTEPH. Additionally, we found that analysis of precordial TWI range can be used to monitor the effectiveness of hemodynamic response to treatment of pulmonary hypertension.

Lack of Relationship between Fibrosis-Related Biomarkers and Cardiac Magnetic Resonance-Assessed Replacement and Interstitial Fibrosis in Dilated Cardiomyopathy.

The relationship between circulating fibrosis-related molecules and magnetic resonance-assessed cardiac fibrosis in dilated cardiomyopathy (DCM) is poorly understood. To compare circulating biomarkers between DCM patients with high and low fibrosis burdens, we performed a prospective, single-center, observational study. The study population was composed of 100 DCM patients (87 male, mean age 45.2 ± 11.8 years, mean ejection fraction 29.7% ± 10.1%). Replacement fibrosis was quantified by means of late gadolinium enhancement (LGE), whereas interstitial fibrosis was assessed via extracellular volume (ECV). Plasma concentrations of cardiotrophin-1, growth differentiation factor-15, platelet-derived growth factor, procollagen I C-terminal propeptide, procollagen III N-terminal propeptide, and C-terminal telopeptide of type I collagen were measured. There were 44% patients with LGE and the median ECV was 27.7%. None of analyzed fibrosis serum biomarkers were associated with the LGE or ECV, whereas NT-proBNP was independently associated with both LGE and ECV, and troponin T was associated with ECV. None of the circulating fibrosis markers differentiated between DCM patients with and without replacement fibrosis, or patients stratified according to median ECV. However, cardiac-specific markers, such as NT-proBNP and hs-TnT, were associated with fibrosis. Levels of circulating markers of fibrosis seem to have no utility in the diagnosis and monitoring of cardiac fibrosis in DCM.

Mortality risk in dilated cardiomyopathy: the accuracy of heart failure prognostic models and dilated cardiomyopathy-tailored prognostic model.

AIMS: The aims of this paper were to investigate the analytical performance of the nine prognostic scales commonly used in heart failure (HF), in patients with dilated cardiomyopathy (DCM), and to develop a unique prognostic model tailored to DCM patients. METHODS AND RESULTS: The hospital and outpatient records of 406 DCM patients were retrospectively analysed. The information on patient status was gathered after 48.2 ± 32.0 months. Tests were carried out to ascertain the prognostic accuracy in DCM using some of the most frequently applied HF prognostic scales (Barcelona Bio-Heart Failure, Candesartan in Heart Failure-Assessment of Reduction in Mortality and Morbidity, Studio della Streptochinasi nell'Infarto Miocardico-Heart Failure, Eplerenone in Mild Patients Hospitalization and Survival Study in Heart Failure, Meta-Analysis Global Group in Chronic Heart Failure, MUerte Subita en Insuficiencia Cardiaca, Organized Program to Initiate Lifesaving Treatment in Hospitalized Patients With Heart Failure, Seattle Heart Failure Model) and one dedicated to DCM, that of Miura et al. At follow-up, 70 DCM patients (17.2%) died. Most analysed scores substantially overestimated the mortality risk, especially in survivors. The prognostic accuracy of the scales were suboptimal, varying between 60% and 80%, with the best performance from Barcelona Bio-Heart Failure and Seattle Heart Failure Model for 1-5 year mortality [areas under the receiver operating curve 0.792-0.890 (95% confidence interval 0.725-0.918) and 0.764-0.808 (95% confidence interval 0.682-0.934), respectively].Based on our accumulated data, a self-developed DCM prognostic model was constructed. The model consists of age, gender, body mass index, symptoms duration, New York Heart Association class, diabetes mellitus, prior stroke, abnormal liver function, dyslipidaemia, left bundle branch block, left ventricle end-diastolic diameter, ejection fraction, N terminal pro brain natriuretic peptide, haemoglobin, estimated glomerular filtration rate, and pharmacological and resynchronisation therapy. This newly created prognostic model outperformed the analysed HF scales. CONCLUSIONS: An analysis of various HF prognostic models found them to be suboptimal for DCM patients. A self-developed DCM prognostic model showed improved performance over the nine other models studied. However, further validation of the prognostic model in different DCM populations is required.

Relationships between Pulmonary Hypertension Risk, Clinical Profiles, and Outcomes in Dilated Cardiomyopathy.

Pulmonary hypertension (PH) in patients with heart failure (HF) contributes to a poorer prognosis. However, in those with dilated cardiomyopathy (DCM), the true prevalence and role of PH is unclear. Therefore, this study aimed to analyze the profile of DCM patients at various levels of PH risk, determined via echocardiography, and its impact on outcomes. The 502 DCM in- and out-patient records were retrospectively analyzed. Information on patient status was gathered after 45.9 ± 31.3 months. Patients were divided into 3 PH-risk groups based on results from echocardiography measurements: low (L, n = 239, 47.6%), intermediate (I, n = 153, 30.5%), and high (H, n = 110, 21.9%). Symptom duration, atrial fibrillation, ventricular tachyarrhythmia, ejection fraction, right atrial area, and moderate or severe mitral regurgitation were found to be independently associated with PH risk. During the follow-up period, 83 (16.5%) DCM patients died: 29 (12.1%) in L, 31 (20.3%) in I, and 23 (20.9%) in H. L-patients had a significantly lower risk of all-cause death (L to H: HR 0.55 (95%CI 0.32-0.98), p = 0.01), while no differences in prognosis were found between I and H. In conclusion, over one in five DCM patients had a high PH risk, and low PH risk was associated with better prognoses.

The burden of atrial fibrillation and its prognostic value in patients with dilated cardiomyopathy.

BACKGROUND: Atrial fibrillation (AF) is the most common arrhythmia in patients with dilated cardiomyopathy (DCM). However, the epidemiology as well as clinical and prognostic significance of AF in DCM are poorly defined. AIMS: We aimed to assess the impact and prognostic value of AF in DCM as well as to investigate the concept of AF­induced DCM. METHODS: Hospital records of 285 patients with DCM from 2012 to 2018 with follow-up were analyzed. RESULTS: Atrial fibrillation was present in 89 patients (31%). They were older, more frequently male, hadhigher body mass index, New York Heart Association class, heart rate (HR), creatinine levels, and larger atria (all P < 0.05) than patients without AF. During follow­up (mean [SD], 35 [24] months), death occurred in 20 of the 82 available patients with AF and 22 of the 188 patients without AF (24% and 12%, respectively; P = 0.007). Atrial fibrillation was independently associated with a worse outcome (hazard ratio, 2.4; 95% CI, 1.3-4.3) and was found to be the major cause of DCM in 21 patients (24%). The diagnostic accuracy of the most optimal predictive model for AF­induced DCM was 0.935 (95% CI, 0.903-0.967). Despite numerical differences, survival was similar in DCM patients with and without AF (P = 0.15). CONCLUSIONS: Almost one­third of patients with DCM had AF. Most of the parameters analyzed differed between patients with and without AF, and AF was found to be an independent prognostic factor of DCM. One­fourth of patients with DCM and AF met the diagnostic criteria for AF­induced DCM.